At operation, a 4.5 cm well-circumscribed solid tumor was found adherent to the spermatic cord.. The tumor consisted of spindle-shaped cells proliferating in short fascicles between nume
Trang 1C A S E R E P O R T Open Access
Angiomyofibroblastoma of the spermatic cord:
a case report
Nikolaos E Tzanakis1, George A Giannopoulos1*, Stamatis P Efstathiou2, Georgios E Rallis1, Nikolaos I Nikiteas3
Abstract
Introduction: Angiomyofibroblastoma is a benign soft tissue tumor with tendency to arise in the vulva
Case presentation: We report a 36-year-old Greek Caucasian man presenting with a left inguinal painless mass This is the second case of angiomyofibroblastoma of the spermatic cord At operation, a 4.5 cm well-circumscribed solid tumor was found adherent to the spermatic cord The tumor consisted of spindle-shaped cells proliferating in short fascicles between numerous medium-sized blood vessels with thin and hyalinized walls Neoplastic cells had eosinophilic cytoplasm with neither mitotic figures nor nuclear atypia The stroma included abundant mast cells and few mature lypocytes Immunostaining showed positivity for vimentin, CD34, desmin and smooth muscle actin Our patient was treated by simple excision and was followed up for five years with clinical examination and ultrasonography, revealing no evidence of local recurrence or metastasis
Conclusion: This unusual neoplasm should be distinguished from aggressive angiomyxoma and other myxoid malignant tumors with widespread metastatic potential
Introduction
In 1992, Fletcheret al [1] described 10 cases of a
pre-viously unrecognized benign soft tissue tumor of the
vulva that was often misdiagnosed as aggressive
angio-myxoma The term angiomyofibroblastoma (AMF) was
endorsed for this novel tumor The morphologic
hall-marks of this tumor were its well-circumscribed
mar-gins, prominent vascularity and features suggestive of
myofibroblastic differentiation [1] Since the
aforemen-tioned original study, there have been several additional
reports of AMF of the genital tracts of both men [2,3]
and women [4,5], but only a single case of this tumor
arising from the spermatic cord [6] The purpose of this
study is to expand the experience with AMF by
describ-ing the second case of the latter unusual location of this
rare lesion and providing a long period of follow-up
Case presentation
A 36-year-old Greek Caucasian man presented with a
left inguinal painless mass that had been growing slowly
for six months During operation, a 4.5 cm
well-circum-scribed solid tumor was found adherent to the spermatic
cord The testis and the epididymis were not involved The lesion was pale gray with a vague lobular and focally glistening cut surface On microscopic examina-tion, the tumor was well-demarcated and consisted of spindle-shaped cells proliferating in short fascicles between numerous medium-sized blood vessels with thin and hyalinized walls (Figure 1) Focally, the tumor cells had an epithelioid appearance with eosinophilic cytoplasm, plump nuclei and neither mitotic figures nor nuclear atypia The stroma included abundant mast cells and few mature lypocytes Immunostaining of neoplastic cells showed intense positivity for vimentin, CD34 and desmin (Figure 2), mild positivity for smooth muscle actin and no staining for keratin and S100 protein Our patient was treated by simple excision and was followed
up for five years with clinical examination and ultraso-nography of the inguinal region revealing no evidence of local recurrence or metastasis
Discussion
AMF is a recently-described soft tissue tumor character-ized by unique morphologic features, a tendency to emanate from the vulva, and a benign biologic beha-viour [1,2] There are few reported cases of occurrence
* Correspondence: geogianno@hotmail.com
1 4th Surgical Department, Attikon Hospital, University of Athens, (1 Rimini
str), Athens, (124 62), Greece
© 2010 Tzanakis et al; licensee BioMed Central Ltd This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in
Trang 2in men The tumors were localized in the scrotum and
the inguinal area of the male patients [4,5,7]
The cell of origin of AMF among males has not been
identified with certainty Regarding the female patients,
it is believed that the tumor is derived from
mesenchy-mal cells in the subepithelial myxoid stromesenchy-mal zone that
extends from the endocervix to the vulva [2], this
hypothesis explaining to some extent the propensity of
this tumor to arise in the lower genital tract All the published studies of patients with AMF have presented benign tumors, without local recurrence or metastatic potential However, the latter possibilities cannot be excluded, since the respective follow up periods were relatively small The above limitation is avoided in the present study, which is the first reporting a sufficiently long follow-up of five years
Figure 1 Moderately cellular area with spindle-shaped tumor cells arranged in short fascicles between numerous vessels with collagenized walls (Hematoxylin-Eosin × 100).
Figure 2 Tumor cells with myofibroblastic morphology intensely positive for desmin (Avidin-Streptavidin method × 200).
Trang 3The most crucial issue is to determine whether this
case should be assigned to AMF or to aggressive
angio-myxoma (AAM) The latter is a histologically benign
soft tissue tumor, associated with a high risk of local
recurrence as well as with local infiltration that often
results in entrapment of nerves and mucosal glands,
thus making complete excision difficult [8] It has been
suggested that AMF and AAM are related neoplasms,
both included in a wide spectrum of angiomyxoid
tumors, which exhibit some overlapping features and
various combinations of myofibroblastic, fibroblastic and
lipomatous differentiation [9] The macroscopic
charac-teristics of our case report are in agreement with those
demonstrated by AMF rather than AAM, since the
tumor was a well-circumscribed, relatively small-sized
lesion with no infiltrating margins [4] Furthermore, the
recognition of cytologic features reminiscent of
myofi-broblastic differentiation is paramount for the diagnosis
of genital AMF and its differentiation from AAM in the
particular patient, whereas intralesional fat tissue as
observed in our case is also more frequently found in
AMF [4]
Nevertheless, although desmin expression was
pre-viously thought to be specific of AMF, this protein is no
longer considered as a reliable marker for distinguishing
the latter from AAM, inasmuch as immunopositivity for
desmin and muscle-specific actin has more recently
been shown in a substantial proportion of AAM [9]
Regarding the surgical management of these rare
neo-plasms (AMF and AAM), the most important factor for
prognosis is the surgical and macroscopic delimitation
of the tumor Because most AMFs have been
success-fully treated with simple excision, this seems to be the
appropriate therapy for these tumors [1-6] After
histo-logical examination, wide excision is meanwhile required
in cases of AAM because of the propensity of the latter
for local recurrence [10]
The differential diagnosis of AMF also includes
smooth muscle tumors, peripheral nerve sheath tumors,
glomus tumor, chondroid syringoma, myxoid malignant
fibrous histiocytoma, angiomyolipoma, spindle cell
lipoma and myxoid liposarcoma The distinction
between AMF and these tumors has been described in
detail elsewhere [1] As most of these diagnoses were
introduced before the original description of AMF, the
aforementioned entities should be easily discriminated
from AMF by routine light microscopic examination in
conjunction with immunohistochemical studies and
electron microscopic examination in selected cases
[2-5] Lastly, the staining of tumor cells in our case with
antibodies to CD34, a 115-kDa transmembrane
glycoprotein associated with cellular interaction and
adhesion, is an additional finding compatible with the diagnosis of AMF [2-5]
Conclusion
Although the exact nosologic position of AMF is still surrounded by some controversy and requires further elucidation, we conclude that our case represents the second report of AMF of the spermatic cord, based on its conventional histopathologic and immunophenotypic features Simple excision appears sufficient for the surgi-cal management of AMF, whereas wide excision after histological examination is needed for the management
of the related AAM, which is associated with a high risk
of local recurrence and infiltration
Consent
Written informed consent was obtained from our patient for publication of this case report and accompa-nying images A copy of the written consent is available for review by the Editor-in-Chief of this journal
Author details
1 4th Surgical Department, Attikon Hospital, University of Athens, (1 Rimini str), Athens, (124 62), Greece.2Department of Internal Medicine, Hygeias Melathron Hospital, (6 Therianou str), Athens, (114 73), Greece 3 2nd Propedeutic Department of Surgery, Laikon Hospital, University of Athens, (17 Agiou Thoma str), Athens, (115 27), Greece.
Authors ’ contributions
NT analyzed and interpreted our patient data, GG, SE and GR contributed equally in designing and writing the paper with NT Meanwhile, NN was involved in drafting the manuscript and revising it critically All authors read and approved the final manuscript.
Competing interests The authors declare that they have no competing interests.
Received: 5 November 2009 Accepted: 4 March 2010 Published: 4 March 2010 References
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doi:10.1186/1752-1947-4-79
Cite this article as: Tzanakis et al.: Angiomyofibroblastoma of the
spermatic cord: a case report Journal of Medical Case Reports 2010 4:79.
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